|Year : 1974 | Volume
| Issue : 4 | Page : 26-27
IN Raizada, VK Pratap, TD Mishra, VK Rohatgi
Department of ophthalmology L. L.R.M. Medical College, Meerut, India
I N Raizada
Department of ophthalmology L. L.R.M. Medical College, Meerut
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Raizada I N, Pratap V K, Mishra T D, Rohatgi V K. Orbital rhabdomyosarcoma. Indian J Ophthalmol 1974;22:26-7
Rhabdomyosarcoma is a primary, highly malignant tumor of the orbit and is probably the most common in the paediatric age group. Prior to 1972, only 14 cases have been recorded in literature by Calhoun and Reese.  Fifteen per cent of all rhabdomyosarcomas occur in the orbit.
The purpose of this paper is to record an interesting case of ocular rhabdomyosarcoma with some unusual features.
| Case Report|| |
I. R. a girl aged 14 years was admitted in the ophthalmic ward with a swelling in the upper outer quadrant of the right orbit, gradually increasing in size and producing progressive painful protrusion of the right eye. Proptosis was directed down and in. She was unconsciously hit by the foot of her younger brother while sleeping, 15 days prior to the onset of the swelling. Family history revealed that the elder sister had died at the age of 15 years of a similar swelling. Systemic examination revealed no features suggestive of malignancy.
Ophthalmological examination revealed that the proptosis was directed down and in. Ocular movements were restricted in all the meridians. Palpebral fissure was narrow, upper lid skin was hyperaemic with visible distended blood vessels. Later the skin of the upper lid got ulcerated and there was bleeding.
A rounded firm, fixed, non-mobile and tender mass 3 cms x 3 cms was palpable under the lid in the upper and outer quadrant of the right orbit. There was no enlargement of pre-auricular glands. Conjunctiva was chemosed. Vision in right eye was counting fingers at 1/2 metre. A course of antibiotics was given and an aspiration was attempted, but was of no avail. [Figure - 1],[Figure - 2]
Fundus examination showed early papilloedema with shallow retinal detachment in the upper temporal quadrant without any holes.
Left eye was normal.
Radiology -Posterio-anterior4view of skull and right lateral view of orbit showed erosion of the roof of the right orbit. A soft tissue shadow was visualized over the right orbit.
Microscopic examination revealed mesenchymal blastoma with varying degrees of maturation of rhabdomyoblastic element Mesenchymal element was composed of loose myxomatous matrix. Several Faint cells were visible [Figure - 3]. PTAH staining revealed prominent cross striations in the rhabdomyoblastic element. At places early cross striations could be appreciated [Figure - 4].
| Discussion|| |
Reese and Calhoun  reported that the average age at which rhabdomyosarcoma occurs is 6-7 years and that the tumor is more frequent in boys than girls. Forest  recorded all four cases under 10 years with equal sex distribution. The upper inner angle of the orbit is the commonest site.
The case in question is a female well beyond the age of 10 years (14 years) and the tumor arose in the upper outer angle of the orbit. The family history was suggestive of heredity. Instances of rhabdomyosarcoma occurring in siblings are very rare  . Onset of proptosis was preceded by trauma. No local or general signs of malignancy were visible.
Fundus showed early papilloedema. Calhoun and Reese have reported it only in one case out of a series of 19. Retinal detach ment is of extremely rare occurrence. Only one case has bcen reported in literature uptil now by Chandra et al  .
| Summary|| |
Clinico-pathological features of an embryonal rhabdomyosarcoma have been discussed. Elder sister of the patient also died of rhabdomyosarcoma.
| References|| |
Calhoun, F and Reese, A.B. 1942, Arch. Ophthal,
Chandra, Ahluwalia and Chugh, 1972, Oriental Arch. of Ophthal, 10,
Forest A. W. 1949, Arch. Ophthal, 12,198.
Howard and Casten, Cited by David D. Donaldson in Atlas of External diseases of the eye. Vol. II. The Mosby Company, St. Louis, 1968. p. 47.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]